Objective:

Our study aims to characterize clinical presentations, radiological findings, management and treatment outcomes of pediatric patients presenting with myelin oligodendrocyte glycoprotein (MOG) associated longitudinally extensive transverse myelitis (LETM).

Background:

Myelin oligodendrocyte associated antibody disorder (MOGAD) is a demyelinating disorder of the central nervous system that demonstrates a wide phenotype, particularly in the pediatric population. Longitudinally extensive transverse myelitis (LETM)-defined as lesions extending ≥ 3 vertebral segments- is a distinct presenting feature of MOGAD.

Design/Methods:

Retrospective review of medical records of pediatric patients (age < 18 years), with a diagnosis of MOGAD associated LETM, between the years 2020 and 2025, at the Children’s Hospital of Pittsburgh.

Results:

A total of 17 patients diagnosed with MOGAD associated LETM were identified. Mean age of presentation was 9 years, with a slight male predominance. Common presenting symptoms included headaches, lower extremity weakness, and gait instability. MRI analyses revealed the highest frequency of lesions within cervical and thoracic cord regions, with a minority involving the lumbar cord. Post-contrast enhancement was seen in a small minority of patients- three with lesional enhancement and one with leptomeningeal enhancement. All patients had concurrent supratentorial brain lesions. Everyone received treatment with high dose steroids, followed by a steroid taper, while seven patients were also treated with IVIG or plasmapheresis. Upon longitudinal follow up, one patient had relapsed; rest showed complete resolution of radiologic findings with minimal to no neurological deficits.

Conclusions:

MOGAD associated LETM in children is characterized by prominent neurologic deficits, a predilection to cervical and thoracic cord, excellent steroid responsiveness, and a low relapse rate. Post-contrast enhancement, longer segments of spine involvement, were shown to indicate a worse presentation with high MOG antibody titers, requiring immunotherapies. Overall, although LETM in MOGAD presents with significant initial disability, long term outcomes are favorable with appropriate treatment and monitoring.